Management of gastroschisis with biliary atresia: A case report

Introduction

Gastroschisis and biliary atresia (BA) are both rare surgical disorders of neonates. While neither condition has a known etiology, there is currently no clear association between the two and the estimated odds of coincidence are 1 in 30, 000, 000 livebirths. Nonetheless, a few published reports have mentioned infants diagnosed with both gastroschisis and BA but usually in the setting of poor outcomes and incomplete descriptions of management decisions.

Case presentation

A 1960-g female with limited prenatal care was born at 35 weeks gestation and was found to have gastroschisis, which was managed with a hand-sewn silo and serial reductions until the abdomen was closed. At 25 days old, she developed acholic stools and rising direct hyperbilirubinemia. Workup was concerning for biliary atresia, which was confirmed with an intraoperative cholangiogram and she underwent Kasai portoenterostomy, which was challenging given intestinal malrotation from gastroschisis. Postoperatively, bilirubin levels normalized and pigmented stools returned. Her course was complicated by a small perforation of the ascending colon requiring exploration and creation of a diverting colostomy on post-operative day 7. However, since that time she has done well with normal liver function now over 9 months after portoenterostomy creation, and is pending colostomy takedown.

Conclusion

Prompt recognition and careful consideration of abnormal intestinal anatomy are necessary in the management of concurrent biliary atresia and gastroschisis, but this can be accomplished with favorable outcomes.